Systemic scleroderma, also called systemic sclerosis (SSc), is a multi-system autoimmune disease characterized by the accumulation of scar tissue in the skin and several internal organs such as the heart, kidney, lungs, and gastrointestinal tract.

Risk factors for systemic scleroderma

Most patients develop systemic scleroderma in their 30s or 40s. The exact cause of the disease is unknown, but it is more common among Caucasians, and up to four times more common among women.

Genetics, exposure to toxins, or previous infections, as well as a family history of autoimmune diseases like lupus or rheumatoid arthritis, are believed to be risk factors for the disease. 

What happens in systemic scleroderma?

Systemic scleroderma is thought to develop as a result of interactions between three disease processes —  faulty fibroblasts, autoimmunity, and vasculopathy or blood vessel disease.

Patients with systemic scleroderma have certain defects in cells called fibroblasts, which produce collagen, a substance the body normally uses to repair wounds. These faulty fibroblasts produce too much collagen, which leads to excessive scarring (fibrosis) in the skin and internal organs.

Systemic scleroderma also is considered an autoimmune disease, in which the body makes antibodies that mistakenly attack healthy tissues. Antibodies normally are produced in order to help the body fight foreign organisms such as bacteria and viruses.

Lastly, patients with systemic scleroderma have damage to their small blood vessels. 

Types of systemic scleroderma

There are two subtypes of systemic scleroderma, limited and diffuse, which are diagnosed based on the extent of skin symptoms. Generally, people with limited systemic scleroderma have skin symptoms limited to the face, arms below the elbows, hands, and fingers with few systemic problems. Diffuse systemic scleroderma patients, on the other hand, experience widespread skin involvement accompanied by internal organ damage.

Systemic scleroderma symptoms

All patients with systemic scleroderma have thick, hard, and shiny skin in the early stages of the disease. As it progresses, the affected areas may become tight and harder to move.

Another symptom frequently seen in people with systemic scleroderma is Raynaud’s phenomenon, a condition causing spasms in the blood vessels of the fingers and toes. This causes extremities to turn white and blue when the patient is in a cold environment or under emotional stress.

Other symptoms of systemic scleroderma include hair loss, calcium deposits under the skin known as calcinosis, small and dilated blood vessels under the skin’s surface called telangiectasias, joint pain, muscle weakness, shortness of breath, dry cough, diarrhea, constipation, difficulty swallowing, acid reflux, bloating, and dry eyes or mouth.

Diagnosing systemic scleroderma

Systemic scleroderma can be diagnosed through the clinical examination of skin changes. Various blood tests also can be used to determine the presence of autoantibodies and to assess underlying organ damage. Chest X-rays, urine analysis, CT scans of the lungs, and echocardiogram can be done to assess the function of different organs.

Treatment and prognosis

There is no cure for systemic scleroderma, but several therapies may help reduce the symptoms and slow progression of the disease. About 70-80 percent of patients live more than 10 years after being diagnosed with systemic scleroderma.

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